A case report of a patient with refractory adult-onset Still’s disease who was successfully treated with tocilizumab over 6 years

Abstract

Interleukin-6 overproduction is pathologically involved in adult onset Still's disease (AOSD). We successfully treated a man with refractory AOSD utilizing tocilizumab. Tocilizumab was discontinued after 15 doses due to intestinal bleeding, but the efficacy was sustained over 21 months. Tocilizumab was readministered safely upon recurrence and showed similar efficacy over 6 years. Corticosteroid and NSAIDs could be discontinued and intestinal bleeding was no more observed. Tocilizumab can be a therapeutic option for AOSD.

Anti‐interleukin‐6 receptor antibody, tocilizumab, for the treatment of autoimmune diseases

“…Pathologically it resembles systemic JIA and is considered to be an adult-onset type of systemic JIA. Again, several case and pilot studies reported that tocilizumab treatment improved clinical symptoms and signs of AOSD patients who had been refractory to conventional treatments [61][62][63][64][65][66][67][68]. The clinical efficacy suggests that tocilizumab may become a first line biologic for the treatment of systemic JIA and AOSD.…”

“…RA (in more than 90 countries worldwide) [36][37][38][39][40][41][42] Castleman's disease (in Japan) [52,53] Systemic and polyarticular JIA (in Japan) [57][58][59] Candidate diseases for tocilizumab therapy: Systemic autoimmune diseases SLE [130] Systemic sclerosis [115] Giant cell arteritis and Takayasu arteritis [90,91] Polymyositis Organ specific autoimmune diseases Crohn's disease [73] Relapsing polychondritis [108] Multiple sclerosis, neuromyelitis optica Acquired hemophilia A [149] Chronic inflammatory diseases Adult-onset Still's disease [61][62][63][64][65][66][67][68] Reactive AA amyloidosis [48][49][50][51] Polymyalgia rheumatica [80,91] RS3PE [85] Spondyloarthritides [100,[102][103][104][105] Behcet's disease Uveitis GVHD [150] Autoinflammatory diseases (TRAPS) [152] Tocilizumab has been approved as a biological drug for the treatment of RA, Castleman's disease and juvenile idiopathic arthritis, and is expected to be applicable to various other autoimmune and inflammatory diseases. Abbreviations: RA, rheumatoid arthritis; JIA, juvenile idiopathic arthritis; SLE, systemic lupus erythematosus; RS3PE, remitting seronegative, symmetrical synovitis with pitti...…”

Tocilizumab Improved both Clinical and Laboratory Manifestations Except for Interleukin-18 in a Case of Multiple Drug-Resistant Adult-Onset Still's Disease

“…Although there are reported cases in which TNF blockers were effective against AOSD, the response rate was not particularly robust. Recently, tocilizumab has been made available clinically and an increasing number of cases have been reported in which it was used successfully against the disease (17)(18)(19)(20)(21)(22). Its response rate is, at present, unclear.…”

Adult-onset Still’s disease

“…Следует отметить, что при этом лабораторный ответ более замедленный, нежели при назначении препаратов, прямо влияющих на провоспалительные цитокины. Так, нормализация лабораторных пока-зателей, включая лейкоцитоз и уро-вень СРБ, происходит в течение не-дели после первой инфузии тоцили-зумаба [93], а полный клинико-ла-бораторный ответ отмечался после 7 инфузий [94]. Сходный быстрый клинический ответ, подтвержден-ный данными ультразвукового ис-следования суставов, описан у боль-ных БСРВ после применения ана-кинры [95].…”